Bottom Line:
Abdominopelvic ultrasonography showed that right-sided intra-abdominal organs (liver, gallbladder) were located on the left while left-sided organs (stomach, spleen) were located on the right.His abdominal aorta was on the right while his inferior vena cava was located on the left.So, an analysis of a relatively simple and non-invasive diagnostic tool such as an electrocardiogram allows for suspicion of a cardiovascular anomaly in a setting of scarce diagnostic resources.

Introduction: Dextrocardia with situs inversus is a rare congenital disease. In patients with this condition, the heart is presented as a mirror image of itself with its apex pointing to the right. The pulmonary and abdominal anatomies are reversed. Dextrocardia with situs inversus occurs at birth but its diagnosis may be in adulthood. This case advances knowledge by graphically describing the unusual electrocardiographic features of dextrocardia in a young adult.

Case presentation: We report a case of a 22-year-old Nigerian man of Yoruba ethnicity who presented himself for preadmission medical test. He had a standard 12-lead electrocardiogram which revealed uncommon features: inversion of P waves in leads I, aVL and aVR; dominantly negative QRS waves in leads I, V1 to V6; reverse R wave progression in chest leads; low voltage in V4 to V6; extreme QRS axis; flattened T waves in V4 to V6 and aVR; and inverted T waves in lead I and aVL. An electrocardiogram diagnosis of dextrocardia was made. The differential diagnosis considered was right ventricular hypertrophy. A cardiovascular examination showed pulse rate of 70 beats per minute, blood pressure of 119/62mmHg, visible cardiac impulse at right precordium, apex beat was located at his fifth right intercostal space mid-clavicular line. A chest X-ray (posterior anterior view) including upper abdomen showed dextrocardia; his aortic arch was located on the right. His stomach bubble was located below his right hemidiaphragm. His trachea was slightly deviated to the left. The findings in his lung fields were not remarkable. Abdominopelvic ultrasonography showed that right-sided intra-abdominal organs (liver, gallbladder) were located on the left while left-sided organs (stomach, spleen) were located on the right. His abdominal aorta was on the right while his inferior vena cava was located on the left. A diagnosis of dextrocardia with situs inversus was made ultrasonographically.

Conclusions: A properly interpreted electrocardiogram was useful in suspecting the diagnosis of dextrocardia with situs inversus. So, an analysis of a relatively simple and non-invasive diagnostic tool such as an electrocardiogram allows for suspicion of a cardiovascular anomaly in a setting of scarce diagnostic resources.

Fig4: Chest X-ray of a 22-year-old Nigerian man with dextrocardia and situs inversus (a) compared with a chest X-ray of a 20-year-old Nigerian man (b) with the heart and abdominal organs normally positioned

Mentions:
An ECG is a graphical record of the electrical activities of a heart obtained on the body surface. It is a basic non-invasive investigation with great application in medical practice [8]. ECG is a cheap, portable and harmless medical test that is very useful in the assessment of cardiac electrical activities and to some extent cardiac structures. ECG was acknowledged as the most commonly conducted cardiovascular diagnostic procedure in clinical practice [9]. Dextrocardia is one of the cardiac anomalies that presents itself with unusual and specific electrocardiographic features which include inversion of P waves in leads I and aVL, dominant S waves in leads I and V1 to V6, reversed R wave progression in chest leads, low voltage QRS axis in V4 to V6, extreme QRS axis, flattened T waves in V4 to V6 and aVR and inverted T waves in lead I and aVL. These findings were present in our patient and were consistent with ECG findings of dextrocardia in previous studies [3, 4]. A comparison between a chest X-ray of our patient (Fig. 4a) and a chest X-ray (Fig. 4b) of another young man of the same age group grossly demonstrated the mirror image nature of dextrocardia. In Nigeria, the incidence of dextrocardia is largely unknown but a few cases have been reported [3, 4, 6]. According to a retrospective chart review at Children’s and Women’s Health Centre of British Columbia in Vancouver, Canada, based on a population-based study of cardiac malformations and outcomes associated with dextrocardia between 1 January 1985 and 31 December 2001, the incidence of dextrocardia was estimated to be 1 in 12,019 pregnancies [10]. Dextrocardia with situs inversus may occur with other features in the respiratory tract. Kartagener syndrome occurs in approximately 25% of individuals with mirror image dextrocardia. This disorder is characterized by dextrocardia with situs inversus, sinusitis and bronchiectasis; it is associated with primary ciliary dyskinesia [2]. Our patient presented with respiratory features suggestive of Kartagener syndrome; chest X-ray findings were not remarkable and the diagnosis was not confirmed because a chest CT scan was not done due to financial constraints. Moreover, echocardiography, an investigation which may reveal an associated cardiac structural or functional lesion, was also delayed because of financial challenges. The genetic basis for dextrocardia with situs inversus is not well understood. Familial tendency to dextrocardia was described by Ibrahim in 2013 in Nigeria [11]. The type of dextrocardia associated with respiratory abnormalities was said to be autosomal recessive; however, Soltan and Li [12] described the cardiac anomaly in a kindred group in which four males were affected which suggested an X-linked recessive inheritance. Both autosomal recessive and X-linked variants have been described [13]. Because of the genetic nature of this cardiac anomaly, many unidentified cases are likely to exist among the general population which may pose diagnostic and management dilemmas to physicians because of unusual presentations at unpredictable moments [14-16]. So, screening of an apparently healthy adult population with ECG may resolve some of these medical enigmas and other asymptomatic cardiovascular disorders.Fig. 4

Fig4: Chest X-ray of a 22-year-old Nigerian man with dextrocardia and situs inversus (a) compared with a chest X-ray of a 20-year-old Nigerian man (b) with the heart and abdominal organs normally positioned

Mentions:
An ECG is a graphical record of the electrical activities of a heart obtained on the body surface. It is a basic non-invasive investigation with great application in medical practice [8]. ECG is a cheap, portable and harmless medical test that is very useful in the assessment of cardiac electrical activities and to some extent cardiac structures. ECG was acknowledged as the most commonly conducted cardiovascular diagnostic procedure in clinical practice [9]. Dextrocardia is one of the cardiac anomalies that presents itself with unusual and specific electrocardiographic features which include inversion of P waves in leads I and aVL, dominant S waves in leads I and V1 to V6, reversed R wave progression in chest leads, low voltage QRS axis in V4 to V6, extreme QRS axis, flattened T waves in V4 to V6 and aVR and inverted T waves in lead I and aVL. These findings were present in our patient and were consistent with ECG findings of dextrocardia in previous studies [3, 4]. A comparison between a chest X-ray of our patient (Fig. 4a) and a chest X-ray (Fig. 4b) of another young man of the same age group grossly demonstrated the mirror image nature of dextrocardia. In Nigeria, the incidence of dextrocardia is largely unknown but a few cases have been reported [3, 4, 6]. According to a retrospective chart review at Children’s and Women’s Health Centre of British Columbia in Vancouver, Canada, based on a population-based study of cardiac malformations and outcomes associated with dextrocardia between 1 January 1985 and 31 December 2001, the incidence of dextrocardia was estimated to be 1 in 12,019 pregnancies [10]. Dextrocardia with situs inversus may occur with other features in the respiratory tract. Kartagener syndrome occurs in approximately 25% of individuals with mirror image dextrocardia. This disorder is characterized by dextrocardia with situs inversus, sinusitis and bronchiectasis; it is associated with primary ciliary dyskinesia [2]. Our patient presented with respiratory features suggestive of Kartagener syndrome; chest X-ray findings were not remarkable and the diagnosis was not confirmed because a chest CT scan was not done due to financial constraints. Moreover, echocardiography, an investigation which may reveal an associated cardiac structural or functional lesion, was also delayed because of financial challenges. The genetic basis for dextrocardia with situs inversus is not well understood. Familial tendency to dextrocardia was described by Ibrahim in 2013 in Nigeria [11]. The type of dextrocardia associated with respiratory abnormalities was said to be autosomal recessive; however, Soltan and Li [12] described the cardiac anomaly in a kindred group in which four males were affected which suggested an X-linked recessive inheritance. Both autosomal recessive and X-linked variants have been described [13]. Because of the genetic nature of this cardiac anomaly, many unidentified cases are likely to exist among the general population which may pose diagnostic and management dilemmas to physicians because of unusual presentations at unpredictable moments [14-16]. So, screening of an apparently healthy adult population with ECG may resolve some of these medical enigmas and other asymptomatic cardiovascular disorders.Fig. 4

Bottom Line:
Abdominopelvic ultrasonography showed that right-sided intra-abdominal organs (liver, gallbladder) were located on the left while left-sided organs (stomach, spleen) were located on the right.His abdominal aorta was on the right while his inferior vena cava was located on the left.So, an analysis of a relatively simple and non-invasive diagnostic tool such as an electrocardiogram allows for suspicion of a cardiovascular anomaly in a setting of scarce diagnostic resources.

Introduction: Dextrocardia with situs inversus is a rare congenital disease. In patients with this condition, the heart is presented as a mirror image of itself with its apex pointing to the right. The pulmonary and abdominal anatomies are reversed. Dextrocardia with situs inversus occurs at birth but its diagnosis may be in adulthood. This case advances knowledge by graphically describing the unusual electrocardiographic features of dextrocardia in a young adult.

Case presentation: We report a case of a 22-year-old Nigerian man of Yoruba ethnicity who presented himself for preadmission medical test. He had a standard 12-lead electrocardiogram which revealed uncommon features: inversion of P waves in leads I, aVL and aVR; dominantly negative QRS waves in leads I, V1 to V6; reverse R wave progression in chest leads; low voltage in V4 to V6; extreme QRS axis; flattened T waves in V4 to V6 and aVR; and inverted T waves in lead I and aVL. An electrocardiogram diagnosis of dextrocardia was made. The differential diagnosis considered was right ventricular hypertrophy. A cardiovascular examination showed pulse rate of 70 beats per minute, blood pressure of 119/62mmHg, visible cardiac impulse at right precordium, apex beat was located at his fifth right intercostal space mid-clavicular line. A chest X-ray (posterior anterior view) including upper abdomen showed dextrocardia; his aortic arch was located on the right. His stomach bubble was located below his right hemidiaphragm. His trachea was slightly deviated to the left. The findings in his lung fields were not remarkable. Abdominopelvic ultrasonography showed that right-sided intra-abdominal organs (liver, gallbladder) were located on the left while left-sided organs (stomach, spleen) were located on the right. His abdominal aorta was on the right while his inferior vena cava was located on the left. A diagnosis of dextrocardia with situs inversus was made ultrasonographically.

Conclusions: A properly interpreted electrocardiogram was useful in suspecting the diagnosis of dextrocardia with situs inversus. So, an analysis of a relatively simple and non-invasive diagnostic tool such as an electrocardiogram allows for suspicion of a cardiovascular anomaly in a setting of scarce diagnostic resources.